2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management Of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (Part 3)
This week, this corner is going to present the results of the meetings of the panels formed by the American College of Rheumatology (ACR).
Before we start, it is best to review the key information which are critical in this column.
The ACR has initiated the move to create guidelines in the management of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAV).
ANCA-AAV is composed of diseases known as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). These diseases affect small and medium-sized vessels and are characterized by multisystem organ involvement.
The ACR has presented the recommendations and ungraded position statements for GPA and MPA
GPA and MPA are identified as different diseases for which disease specific management approaches should be considered.
However, many recommendations and unverified position statements consider GPA and MPA as the same, because important trials have enlisted both groups and presented results for these diseases at the same time.
Therefore, if experts present recommendations and unverified position statements applicable to both GPA and MPA as well as recommendations and unverified position information only applicable to GPA. All recommendations for GPA/MPA are can be considered as conditional, due in part to a lack of multiple randomized controlled trials providing support to the recommendations.
The studies are given a second look to create a body of recommendations which is backed by pieces of evidence.
Understanding that these diseases create an impact to multiple organ systems, the cooperation among rheumatologists, nephrologists, pulmonologists, and otolaryngologists may improve the health of patients suffering from GPA and MPA.
Thus, we now move towards discussing the key recommendations which are formulated by the ACR’s panels.
For patients with active, severe GPA/MPA, it is conditionally recommended to provide treatment with rituximab over cyclophosphamide.
Both rituximab and cyclophosphamide, in combination with glucocorticoids, are used for remission induction in GPA and MPA. Rituximab is known to provide benefits just like cyclophosphamide for remission induction in a randomized controlled trial. Although cyclophosphamide doses should be lower than previous regimens in order to gain the result of less toxicity per treatment course, rituximab is still preferred, since it is considered less toxic than cyclophosphamide.
A single dose of cyclophosphamide may carry huge risks such as neutropenia, bladder injury, and the small potential for infertility.
We will continue next week with the discussion of the other recommendations crafted by the ACR.